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Pediatric Thymoma Follow-up

  • Author: Richard A Bickel, MD; Chief Editor: Harumi Jyonouchi, MD  more...
 
Updated: Mar 21, 2016
 

Complications

Complications may include the following:

  • Structural problems, such as compression syndromes that involve the bronchi or lungs or superior vena cava syndrome (SVCS), can occur from local spread of benign thymoma, from thymic cysts, or from thymic carcinoma. Presenting symptoms may include chest pain, SVCS, dyspnea, dysphagia, and cough.
  • Areas of benign thymoma can become highly vascular or necrotic and lead to bleeding.
  • Clinical manifestations include paraneoplastic syndromes and immunodeficiency.
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Prognosis

Adverse predictive factors include the following:[43, 44]

  • Invasive or metastatic tumor
  • Tracheal or vascular compression
  • Age younger than 30 years
  • Epithelial or mixed histology
  • Tumor size of more than 8 cm [45]

The presence of myasthenia gravis (MG) with thymomas is no longer considered a poor prognostic factor and is actually thought to be a favorable prognostic factor.[46]

Based on WHO classification, the 5-year and 10-year survival rates are as follows:[47, 48]

  • Type A - 100% and 95%, respectively
  • Type AB - 93% and 90%, respectively
  • Type B1 - 89% and 85%, respectively
  • Type B2 - 82% and 71%, respectively
  • Type B3 - 71% and 40%, respectively
  • Type C - 23% (5-year survival rate)

Recurrence of thymoma can occur after resection. A study surgical outcomes after recurrence of thymic epithelial tumors in 67 patients showed an overall survival rate at 10 years of 70% in those undergoing re-resection.[32]

For patients with Masaoka stage IVA thymomas, a study of 18 patients reported survival rates at 3 years (91%), 5 years (78%), and 10 years (65%).[49] These patients underwent multimodality therapy, including surgical resection, preoperative chemotherapy, and even postoperative radiation therapy (in select patients).

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Contributor Information and Disclosures
Author

Richard A Bickel, MD Chief, Allergy Clinic, Moncrief Army Community Hospital

Richard A Bickel, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American College of Allergy, Asthma and Immunology

Disclosure: Nothing to disclose.

Coauthor(s)

Cecilia P Mikita, MD, MPH Associate Program Director, Allergy-Immunology Fellowship, Associate Professor of Pediatrics and Medicine, Uniformed Services University of the Health Sciences; Staff Allergist/Immunologist, Walter Reed National Military Medical Center

Cecilia P Mikita, MD, MPH is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Allergy, Asthma and Immunology

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

David J Valacer, MD 

David J Valacer, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association for the Advancement of Science, American Thoracic Society, New York Academy of Sciences

Disclosure: Nothing to disclose.

Chief Editor

Harumi Jyonouchi, MD Faculty, Division of Allergy/Immunology and Infectious Diseases, Department of Pediatrics, Saint Peter's University Hospital

Harumi Jyonouchi, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association of Immunologists, American Medical Association, Clinical Immunology Society, New York Academy of Sciences, Society for Experimental Biology and Medicine, Society for Pediatric Research, Society for Mucosal Immunology

Disclosure: Nothing to disclose.

Additional Contributors

Terry W Chin, MD, PhD Associate Clinical Professor, Department of Pediatrics, University of California, Irvine, School of Medicine; Associate Director, Cystic Fibrosis Center, Attending Staff Physician, Department of Pediatric Pulmonology, Allergy, and Immunology, Memorial Miller Children's Hospital

Terry W Chin, MD, PhD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Allergy, Asthma and Immunology, American College of Chest Physicians, American Federation for Clinical Research, American Thoracic Society, California Society of Allergy, Asthma and Immunology, California Thoracic Society, Clinical Immunology Society, Los Angeles Pediatric Society, Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author Marion Johnson, MD, to the development and writing of this article.

References
  1. Stites DP. Diseases of the thymus. Wyngaarden J, Smith LH, eds. Cecil Textbook of Medicine. WB Saunders Co; 1988.

  2. Rich RR. Clinical Immunology, Principles and Practice. 2nd ed. Mosby; 1996.

  3. Roitt IM, Brostoff J, Male DK. Immunology. 5th ed. Mosby; 1998.

  4. Temes R, Chavez T, Mapel D, et al. Primary mediastinal malignancies: findings in 219 patients. West J Med. 1999 Mar. 170(3):161-6. [Medline].

  5. Agarwala SS. Paraneoplastic syndromes. Med Clin North Am. 1996 Jan. 80(1):173-84. [Medline].

  6. Mygland A, Vincent A, Newsom-Davis J, et al. Autoantibodies in thymoma-associated myasthenia gravis with myositis or neuromyotonia. Arch Neurol. 2000 Apr. 57(4):527-31. [Medline].

  7. Fauci AS, Braunwald E, Isselbacher KJ. Harrison’s Principles of Internal Medicine. 14th ed. McGraw-Hill; 1998.

  8. Hart IK. Acquired neuromyotonia: a new autoantibody-mediated neuronal potassium channelopathy. Am J Med Sci. 2000 Apr. 319(4):209-16. [Medline].

  9. Evoli A, Minicuci GM, Vitaliani R, et al. Paraneoplastic diseases associated with thymoma. J Neurol. 2007 Jun. 254(6):756-62. [Medline].

  10. Takamori M, Komai K, Iwasa K. Antibodies to calcium channel and synaptotagmin in Lambert-Eaton myasthenic syndrome. Am J Med Sci. 2000 Apr. 319(4):204-8. [Medline].

  11. Ammann AJ, Stiehm ER, Roberts RL. Antibody (B-cell) immunodeficiency disorders. Stites DP, Terr A, eds. Medical Immunology. Appleton & Lange; 1997.

  12. Casadevall N, Dupuy E, Molho-Sabatier P, et al. Autoantibodies against erythropoietin in a patient with pure red-cell aplasia. N Engl J Med. 1996 Mar 7. 334(10):630-3. [Medline].

  13. Frank K, Austin M. Samter’s Immunologic Diseases. Little Brown & Co; 1995.

  14. Gay CM, William WN Jr, Wang SA, Oo TH. Thymoma complicated by acquired amegakaryocytic thrombocytopenia and pure red cell aplasia. J Natl Compr Canc Netw. 2014 Nov. 12(11):1505-9. [Medline].

  15. Strollo DC, Rosado de Christenson ML, Jett JR. Primary mediastinal tumors. Part 1: tumors of the anterior mediastinum. Chest. 1997 Aug. 112(2):511-22. [Medline].

  16. Stiehm ER. Immunologic disorders in infants and children. Ochs HD, Winkelstein J, eds. Disorders of the B-Cell System. 4th ed. 1996. 320.

  17. Watts RG, Kelly DR. Fatal varicella infection in a child associated with thymoma and immunodeficiency (Good's syndrome). Med Pediatr Oncol. 1990. 18(3):246-51. [Medline].

  18. Middleton E, Reed CE, Ellis EF, et al, eds. Allergy: Principles and Practice. 5th ed. Mosby; 1998.

  19. Loehrer PJ Sr. Current approaches to the treatment of thymoma. Ann Med. 1999 Oct. 31 Suppl 2:73-9. [Medline].

  20. Takeda S, Miyoshi S, Akashi A. Clinical spectrum of primary mediastinal tumors: a comparison of adult and pediatric populations at a single Japanese institution. J Surg Oncol. 2003 May. 83(1):24-30. [Medline].

  21. Takanami I, Takeuchi K, Naruke M. Noninvasive large thymoma with a natural history of twenty-one years. J Thorac Cardiovasc Surg. 1999 Dec. 118(6):1134-5. [Medline].

  22. Bonilla FA, Bernstein IL, Khan DA, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. Ann Allergy Asthma Immunol. 2005 May. 94(5 Suppl 1):S1-63. [Medline].

  23. Kelleher P, Misbah SA. What is Good's syndrome? Immunological abnormalities in patients with thymoma. J Clin Pathol. 2003 Jan. 56(1):12-6. [Medline].

  24. Camera L, Brunetti A, Romano M, et al. Morphological imaging of thymic disorders. Ann Med. 1999 Oct. 31 Suppl 2:57-62. [Medline].

  25. Lastoria S, Palmieri G, Muto P, Lombardi G. Functional imaging of thymic disorders. Ann Med. 1999 Oct. 31 Suppl 2:63-9. [Medline].

  26. Panitch HS. Neurologic diseases. Stites DP, Terr AI, Parslow TG, eds. Medical Immunology. Appleton & Lange; 1997.

  27. Muller-Hermelink HK, Marx A. Pathological aspects of malignant and benign thymic disorders. Ann Med. 1999 Oct. 31 Suppl 2:5-14. [Medline].

  28. Bott-Kothari T, Aron BS, Bejarano P. Malignant thymoma with metastases to the gastrointestinal tract and ovary: a case report and literature review. Am J Clin Oncol. 2000 Apr. 23(2):140-2. [Medline].

  29. Das A, Pandit S, Choudhury S, Das SK, Basuthakur S. Superior vena caval syndrome and ipsilateral pleural effusion: A rare presentation of anterior mediastinal thymoma. Lung India. 2014 Oct. 31(4):383-6. [Medline].

  30. Kim DJ, Yang WI, Choi SS. Prognostic and clinical relevance of the World Health Organization schema for the classification of thymic epithelial tumors: a clinicopathologic study of 108 patients and literature review. Chest. 2005 Mar. 127(3):755-61. [Medline].

  31. [Guideline] NCCN Clinical Practice Guidelines in Oncology. Thymomas and Thymic Carcinomas. V.2.2012. 11/9/2011. [Full Text].

  32. Okumura M, Shiono H, Inoue M, et al. Outcome of surgical treatment for recurrent thymic epithelial tumors with reference to world health organization histologic classification system. J Surg Oncol. 2007 Jan 1. 95(1):40-4. [Medline].

  33. Werneck LC, Cunha FM, Scola RH. Myasthenia gravis: a retrospective study comparing thymectomy to conservative treatment. Acta Neurol Scand. 2000 Jan. 101(1):41-6. [Medline].

  34. Raschal S, Siegel JN, Huml J, Richmond GW. Hypogammaglobulinemia and anemia 18 years after thymoma resection. J Allergy Clin Immunol. 1997 Dec. 100(6 Pt 1):846-8. [Medline].

  35. Detterbeck FC, Zeeshan A. Thymoma: current diagnosis and treatment. Chin Med J (Engl). 2013. 126(11):2186-91. [Medline].

  36. Davenport E, Malthaner RA. The role of surgery in the management of thymoma: a systematic review. Ann Thorac Surg. 2008 Aug. 86(2):673-84. [Medline].

  37. Fornasiero A, Daniele O, Ghiotto C, et al. Chemotherapy of invasive thymoma. J Clin Oncol. 1990 Aug. 8(8):1419-23. [Medline].

  38. Macchiarini P, Chella A, Ducci F, et al. Neoadjuvant chemotherapy, surgery, and postoperative radiation therapy for invasive thymoma. Cancer. 1991 Aug 15. 68(4):706-13. [Medline].

  39. Loehrer PJ Sr, Chen M, Kim K, et al. Cisplatin, doxorubicin, and cyclophosphamide plus thoracic radiation therapy for limited-stage unresectable thymoma: an intergroup trial. J Clin Oncol. 1997 Sep. 15(9):3093-9. [Medline].

  40. Venuta F, Rendina EA, Pescarmona EO, et al. Multimodality treatment of thymoma: a prospective study. Ann Thorac Surg. 1997 Dec. 64(6):1585-91; discussion 1591-2. [Medline].

  41. Kunitoh H, Tamura T, Shibata T, Takeda K, Katakami N, Nakagawa K, et al. A phase II trial of dose-dense chemotherapy, followed by surgical resection and/or thoracic radiotherapy, in locally advanced thymoma: report of a Japan Clinical Oncology Group trial (JCOG 9606). Br J Cancer. June 29, 2010. 103(1):6-11. [Medline].

  42. Palmieri G, Lastoria S, Montella L, et al. Role of somatostatin analogue-based therapy in unresponsive malignant thymomas. Ann Med. 1999 Oct. 31 Suppl 2:80-5. [Medline].

  43. Verley JM, Hollmann KH. Thymoma. A comparative study of clinical stages, histologic features, and survival in 200 cases. Cancer. 1985 Mar 1. 55(5):1074-86. [Medline].

  44. Pollack A, Komaki R, Cox JD. Thymoma: treatment and prognosis. Int J Radiat Oncol Biol Phys. 1992. 23(5):1037-43. [Medline].

  45. Wright CD, Wain JC, Wong DR. Predictors of recurrence in thymic tumors: importance of invasion, World Health Organization histology, and size. J Thorac Cardiovasc Surg. 2005 Nov. 130(5):1413-21. [Medline].

  46. Kondo K, Monden Y. Thymoma and myasthenia gravis: a clinical study of 1,089 patients from Japan. Ann Thorac Surg. 2005 Jan. 79(1):219-24. [Medline].

  47. Park MS, Chung KY, Kim KD. Prognosis of thymic epithelial tumors according to the new World Health Organization histologic classification. Ann Thorac Surg. 2004 Sep. 78(3):992-7; discussion 997-8. [Medline].

  48. Rena O, Papalia E, Maggi G. World Health Organization histologic classification: an independent prognostic factor in resected thymomas. Lung Cancer. 2005 Oct. 50(1):59-66. [Medline].

  49. Huang J, Rizk NP, Travis WD, et al. Feasibility of multimodality therapy including extended resections in stage IVA thymoma. J Thorac Cardiovasc Surg. 2007 Dec. 134(6):1477-83; discussion 1483-4. [Medline].

  50. Curran WJ Jr, Kornstein MJ, Brooks JJ, Turrisi AT 3rd. Invasive thymoma: the role of mediastinal irradiation following complete or incomplete surgical resection. J Clin Oncol. 1988 Nov. 6(11):1722-7. [Medline].

  51. Goldman L, Cecil RL, Bennett JC, eds. Cecil Textbook of Internal Medicine. WB Saunders Co; 2000.

  52. Graeber GM, Tamim W. Current status of the diagnosis and treatment of thymoma. Semin Thorac Cardiovasc Surg. 2000 Oct. 12(4):268-77. [Medline].

  53. Inoue M, Okumura M, Miyoshi S, et al. Impaired expression of MHC class II molecules in response to interferon- gamma (IFN-gamma) on human thymoma neoplastic epithelial cells. Clin Exp Immunol. 1999 Jul. 117(1):1-7. [Medline].

  54. Monden Y, Nakahara K, Iioka S, et al. Recurrence of thymoma: clinicopathological features, therapy, and prognosis. Ann Thorac Surg. 1985 Feb. 39(2):165-9. [Medline].

  55. Spigland N, Di Lorenzo M, Youssef S, et al. Malignant thymoma in children: a 20-year review. J Pediatr Surg. 1990 Nov. 25(11):1143-6. [Medline].

  56. Stephan JL, Galambrun C, Boucheron S, et al. Epstein-Barr virus--positive undifferentiated thymic carcinoma in a 12- year-old white girl. J Pediatr Hematol Oncol. 2000 Mar-Apr. 22(2):162-6. [Medline].

 
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Table 1. Comparison of the Different Classifications of Thymic Epithelial Tumors [27]
Clinicopathologic Classification WHO Type Terminology of the Histogenetic Classification for the Histologic Subtypes of Thymic Epithelial Tumors
Benign thymoma A



AB



Medullary thymoma



Mixed thymoma



Malignant thymomas,



Category I



B1



B2



B3



Predominantly cortical thymoma



Cortical thymoma



Well-differentiated thymic carcinoma



Malignant thymomas,



Category II



C Epidermoid keratinizing (squamous cell) carcinoma



Epidermoid nonkeratinizing carcinoma



Lymphoepithelioma-like carcinoma



Sarcomatoid carcinoma (carcinosarcoma)



Clear cell carcinoma



Mucoepidermoid carcinoma



Undifferentiated carcinoma



Table. Macchiarini et al (1991) [38]
Cisplatin 75 mg/m2 on day 1 3 courses repeated q3wk
Epirubicin 100 mg/m2 on day 1
Etoposide 120 mg/m2 on days 1, 3, and 5
Surgery and radiation



in patients with



complete or partial



response to chemotherapy



4500 cGy if complete



resection



6000 cGy if incomplete



resection



 
Table. Loehrer et al (1997) [39]
Cisplatin 50 mg/m2 2-4 cycles q3wk
Doxorubicin 50 mg/m2
Cyclophosphamide 500 mg/m2
Followed by radiation 54 Gy to the primary tumor and lymph nodes
Table. Venuta et al (1997) [40]
Cisplatin 75-100 mg/m2 on day 1 Repeated q3wk 3 times before surgery and 2 or 3 times after surgery
Epirubicin hydrochloride 100 mg/m2 on day 1
Etoposide 120 mg/m2 on days 1, 3, and 5
Postoperative radiation in patients with radical resection 30 Gy Delivered in 3 wk with 5 fractions per wk
Postoperative radiation in



patients with incomplete resection



50 Gy Delivered in 5 wk with 5 fractions per wk
Table. Palmieri et al (1999) [42]
Octreotide 1.5 mg/d SC In patients shown to have



somatostatin receptors



Lanreotide 30 mg/d SC q14d Switch to this longer-acting



somatostatin analogue or depot form of octreotide if short-acting octreotide



is well tolerated



Prednisone 0.6 mg/kg/d PO



for 3 mo, then



decreasing to 0.2 mg/kg



 
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